A 16-year-old girl was admitted to a hospital after having noticed clearly palpable abdominal mass, without accompanying symptoms. At surgery a tumour superficially attached to the pancreatic tail, well-encapsulated, measuring approx. 8x5x3 cm, had been found and resected. Histological examination using routine hematoxylin and eosin staining, additional histochemical and immunohistochemical techniques revealed low-grade tumour with mixed appearances of PSEN and pancreatoblastoma; both of these tumours originate from pancreatic primordia.